title: Congenital dyserythropoietic anaemias: splenectomy as a mode of therapy.
abstract: 3 cases of congenital dyserythropoietic anaemia (type I, case 3; type III, cases 1 and 2) are reported with a follow-up of 8-10 years. Conventional therapy with vitamins, steroids and/or anabolic hormones was ineffective. Regular blood transfusions were required to maintain the haemoglobin levels around 8 g/dl. Splenectomy, however, resulted in a sustained rise in haemoglobin without blood transfusions. During the follow-up period varying from 40 to 77 months after splenectomy, diabetes mellitus was detected in all 3 patients. Liver biopsy showed haemosiderosis in 2 patients with cirrhosis in one of them. It is suggested that splenectomy could be an effective mode of therapy to reduce the need for blood transfusion in congenital dyserythropoietic anaemias.
mesh_terms: D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood / Q000188:drug therapy / Q000628:therapy*; D000745:Anemia, Hemolytic, Congenital / Q000628:therapy*; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D004247:DNA / Q000096:biosynthesis; D048909:Diabetes Complications; D006410:Hematopoiesis; D006486:Hemosiderosis / Q000150:complications; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications; D008297:Male; D008938:Mitosis; D011239:Prednisolone / Q000627:therapeutic use; D013156:Splenectomy
chemical_list: D004247:DNA; D011239:Prednisolone